Rare form of cancer has high survival rate

Ulysses Torassa, Chronicle Staff Writer

Published 4:00 am, Tuesday, August 3, 2004

Neuroendocrine tumors, the kind that Apple Chief Executive Officer Steve Jobs had surgically removed during the weekend, fall into a tiny category of pancreatic cancers that tend to grow very slowly and have a high survival rate, medical experts said Monday.

Jobs' form of the disease accounts for less than 5 percent of the 32,000 pancreatic cancer cases diagnosed each year in the United States.

Dr. Jeffrey Norton, a specialist in cancers of the digestive system at Stanford University Medical Center, said an estimated 80 to 90 percent of patients are still alive after 10 years, although predictions are difficult because the number of cases is so small.

The far more common adenocarcinoma variety kills 95 percent of patients within five years. Many people die just three to six months after diagnosis, although recently there have been stepped-up efforts to find better treatments and improve the dismal survival rates.

Doctors don't know exactly why the two kinds of cancer behave so differently. But molecular studies have shown that neuroendocrine tumors (also known as islet cell tumors) have fewer genetic mutations than adenocarcinomas, which may help explain the different pace of disease progression.

Because neuroendocrine tumors grow so slowly, chemotherapy and radiation, which attack fast-growing cells, usually aren't part of the treatment.

"They key is to remove it surgically," Norton said. "If it can be completely removed surgically, we're talking about a good prognosis."

The pancreas, a tube-shaped organ tucked behind the stomach, secretes hormones and enzymes important in digestion. In adenocarcinoma, cancer arises in the cells lining the pancreatic ducts. Neuroendocrine tumors occur in different areas, affecting cells that produce substances such as insulin, glucagon and a host of lesser-known players in the body's food-processing system. Neuroendocrine tumors are further categorized as functioning or nonfunctioning, with functioning tumors leading to a range of symptoms, from excessive hunger to telltale rashes and stomach ulcers.

Jobs' cancer apparently fell into the nonfunctioning category, which produce few symptoms. If the tumor gets large enough, it can cause obstructions or bleeding, but it is often found earlier by accident when imaging studies like CT scans or MRIs are taken of a person's torso for another reason, Norton said.

In his e-mail to Apple employees announcing his diagnosis, Jobs did not say how doctors discovered the cancer. An Apple spokesman would not release further details about circumstances surrounding Jobs' treatment. Jobs is also chief executive of Emeryville's Pixar Animation Studios.

Julie Fleshman, president and CEO of the Pancreatic Cancer Action Network, hopes the publicity surrounding Jobs' illness will help create more interest in the disease, which until recently has been a relatively low priority for federal research funds. "Just today the flood of e-mails I've received has been incredible," she said. "The more attention there is about the disease, the more that fuels research, and eventually a cure."

The association hosts a Web site, www.pancan.org, and fields calls from patients on a toll-free number, (877) 272-6226.

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